Mouse models of mitochondrial complex I dysfunction

摘要: Diseases of the mitochondria generally affect cells with high-energy demand, and appear to most profoundly excitatory that have localized high energy requirements, such as neurons cardiac skeletal muscle cells. Complex I mammalian mitochondrial respiratory chain is a very large, 45 subunit enzyme, functional deficiency complex frequently observed cause oxidative phosphorylation (OXPHOS) disorders. Impairment results in decreased cellular production responsible for variety human encephalopathies, myopathies cardiomyopathies. may be caused by mutations any seven or 38 nuclear genes encode subunits various other assembly function. Mouse models faithfully mimic disorders are needed better understand role health disease evaluation potential therapies diseases. In this review we discuss existing mouse dysfunction, focusing on those similarities We also some noteworthy murine genetic which not disrupted, but dysfunction observed, along more popular chemical compounds inhibit function useful modeling mice. This article part Directed Issue entitled: Bioenergetic adaptation therapy.